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1 Jared Bozeman Kathleen Luskin MD Bipin Thapa MD Medical College of Wisconsin Milwaukee, Wisconsin Nothing to disclose 24 Year old previously healthy woman presenting from OSH Fatigue Weakness Neck swelling Unintentional weight loss of 20 pounds Hand sores Gradual over 5 weeks 1
2 PMH: TMJ dysfunction PSH: none No known exposures Family HX: no cancers or autoimmune disorders Meds: NSAIDS prn Social history House keeper at local hotel Denied ETOH, tobacco or ilicit drug use Sexually active with boyfriend, used condoms Transferred to academic medical center after 1 week Cervical lymph node biopsy CD15+, CD30- cells Necrotizing lymphadenitis Corticosteroid treatment initiated D/C due to lack of clear etiology BP: 115/64 mmhg Temperature: 100 F (spiking to 103 F) Pulse: 68 bpm RR 18 bpm SpO2 96% 2
3 General: uncomfortable but no acute distress Lymph: posterior cervical lymphadenopathy, parotid swelling GI: absent hepatosplenomegaly Skin: numerous erythematous, nonpainful/tender plaques and pustules on hands 3
4 Kikuchi Disease Sarcoidosis Sjögren s syndrome CREST Syndrome Mixed connective tissue disease Tuberculosis Lymphoma Hepatitis C Scleroderma Hb: 8.5 g/dl WBC: 2.5 x 10 3 /mm 3 ANC: 1.62 x 10 3 /mm 3 (L) ALC: 0.63 x 10 3 /mm 3 (L) PLT: 266 BCX, HIV, mumps, EBV, CMV: negative HCV AB: positive 4
5 K: 3.3 mmol/l ALB: 3.2 g/dl AST: 41 U/L ALT: 35 U/L ANA: positive (index 2.28) SCL 70: positive Anti dsdna AB: negative ESR 108 Normal complement levels CT of neck: hyperplasia of bilateral parotid glands and bilateral LAD cervical nodes CT of Abdomen: benign, no HSM Punch biopsy of skin lesions Immune complex deposition Bone marrow biopsy Decreased hematopoiesis No significant dysplasia Review of outside slides Negative for infectious etiology 5
6 Necrotizing lymphadenitis Rare in SLE (Shrestha et al) But not impossible (Wallace et al) The negative dsdna antibodies Specific, not sensitive for SLE (Isenberg et al) Status of HCV Infection vs. inflammatory red herring CD15+ cells Malignancy concern Rare, benign necrotizing lymphadenitis Idiopathic Speculation autoimmune Young, Asian women Symptoms overlap with SLE Fever Weight loss Generally self resolving Results of skin biopsy Classic for Type III autoimmune disease Clinical diagnosis Nasal ulcers ANA positive Evidence of chronic inflammatory state Rash 6
7 Diagnosed with SLE Initiated on corticosteroids and hydroxychlorquine Improvement of hand lesions Subjective improvement in symptoms Following with rheumatology clinic Serositis Oral (nasal) ulcers Arthritis Photosensitive rash Blood dyscrasia Renal abnormalities ANA positivity Immunological phenomenon Neurological symptoms Malar rash Discoid lesions American College of Rheumatology 4 of 11 required SOAP BRAIN MD Khanna D, Shrivastava A, Malur PR, Kangle R. Necrotizing Lymphadenitis in Systemic Lupus Erythematosus: Is it Kikuchi-Fujimoto Disease? JCR J Clin Rheumatol. 2010;16(3). Available at: ymphadenitis_in_systemic_lupus.5.aspx. Daniel J Wallace M, David S Pisetsky, MD P, Monica Ramirez Curtis, MD M. Diagnosis and differential diagnosis of systemic lupus erythematosus in adults. UpToDate.com. Available at: Isenberg DA, Manson JJ, Ehrenstein MR, Rahman A. Fifty years of anti-ds DNA antibodies: are we approaching journey s end? Rheumatol. 2007;46 (7 ): doi: /rheumatology/kem112. Shrestha D, Dhakal AK, KC SR, Shakya A, Shah SC, Shakya H. Systemic lupus erythematosus and granulomatous lymphadenopathy. BMC Pediatr. 2013;13:179. doi: / Alguire, Patrick et al Internal Medicine Essentials for Students: a Companion for MKSAP for Students 5. American College of Physicians. Philadelphia, PA
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